Approach Considerations

Management of iris juvenile xanthogranuloma (JXG) should be prompt to avoid the development of iritis with photophobia, advanced hyphema, secondary glaucoma, neovascularization-related complications, loss of vision, and, even, loss of the eye.

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Medical Care

Pharmacotherapy: Topical, subconjunctival, intralesional, and systemic corticosteroids are used for intraocular and orbital juvenile xanthogranuloma (JXG). Orbital lesions may respond to intralesional steroid injections. Iris lesions are treated with topical prednisolone or subconjunctival steroids. These are followed by sub-Tenon steroids if no improvement occurs in several weeks.

The first-line treatment of iris JXG is high-dose corticosteroids applied topically (every two hours during waking hours and ointment at night) with a slow taper. If there is difficulty administering topical medication, periocular steroids can be considered, although the effects of the steroids may last for 3-4 months.

Systemic steroids are considered only if the topical steroids do not yield an adequate response.

Radiotherapy: Low-dose radiation may be the treatment of choice for diffuse uveal lesions, especially if glaucoma is present, or if there is poor response to steroid treatment. Typically, 100-200 cGy is administered per dose over a 2- to 3-week period. The total is usually kept under 500 cGy, but higher doses are used for poorly responsive tumors.

Antimetabolites are sometimes used as adjuvants to radiotherapy.

Appropriate glaucoma medications should be used in the setting of hyphema and increased intraocular pressure.

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Surgical Care

Occasionally, surgery is indicated for localized iris lesions involving less than one quadrant. However, the risks to normal ocular structures may outweigh the benefits.

The surgeon should be prepared to deal with massive amounts of bleeding, which can occur with iridectomy in this setting.

Conjunctival, eyelid, and orbital JXG may be resected or treated with steroids. Topical corticosteroids may be used for limbal JXG and intralesional steroids for eyelid JXG, although surgical resections are also reasonable. Similarly, solitary orbital JXG can be surgically resected. Kontos et al have shown that spontaneous regression of corneoscleral limbus xanthogranulomas is unlikely and recommend prompt surgical intervention.
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Consultations

Discussion with a pediatric dermatologist or radiation oncologist may be helpful to document the extent of the disease and plan treatment.

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Diet

No dietary restrictions are indicated for this disorder.

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Activity

Standard activity restrictions should be put in place for patients who develop spontaneous hyphema. These restrictions generally include bed rest with bathroom privileges and elevation of the head of the bed 30° from the horizontal.

Small children should have a protective shield over the eye at all times to prevent rubbing.

Eye protection, especially during sports, should be prescribed for older children who have known ocular tumors.

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Contributor Information and Disclosures

Author

Bhupendra C K Patel, MD, FRCS Professor of Ophthalmic Plastic and Facial Cosmetic Surgery, Department of Ophthalmology and Visual Sciences, John A Moran Eye Center, University of Utah School of Medicine